Autologous Intestinal Transplant Saves the Day for Gardner's Syndrome Patient

Action Points

Note that bowel transplant has typically been done using cadaver tissue.

Inform interested patients that small intestine auto-transplant requires viable, uninvolved bowel and is typically not done when there is a risk of metastasis.

CHICAGO, Feb. 7 -- The desmoid tumor was back and bigger than ever, despite the multiple surgeries that had claimed his colon and part of his small intestine. William Crook had one chance left at a functional outcome.

He turned to Enrico Benedetti, M.D., of the University of Illinois Medical Center here.

Dr. Benedetti removed the 11-pound tumor and performed one of the first small intestine auto-transplants, preserving Crook's ability to eat and to digest food.

Crook has Gardner's syndrome, a rare genetic disorder that causes a proliferation of polyps that often lead to cancer. While desmoid tumors do not metastasize, they tend to recur and their aggressive growth can be life threatening.

A limited resection in 2004 had failed to put the brakes on the tumor's growth. Crook couldn't eat, was on narcotics for the excruciating pain, and thought he was dying.

Then his family heard of Dr. Benedetti's experience with bowel transplant.

Dr. Benedetti and colleagues at the University of Illinois Medical Center have performed 24 of the 42 living-donor small intestine transplants reported worldwide.

Bowel transplants have traditionally been done using cadaver tissue, but in 1998, Dr. Benedetti started using bowel segments from live donors, typically a human leukocyte antigen (HLA)-matched parent or sibling.

"The main consideration was the ability to intervene sooner and the potential advantage from an immunological standpoint of using a closely related donor," Dr. Benedetti said.

Six of the cases were combined liver and bowel living-donor transplants in children, most of whom were affected by short bowel syndrome. These young children had a 30% chance of dying on the waiting list otherwise.

As Dr. Benedetti and colleagues reported in the Annals of Surgery in 2006, all donors have recovered well and left the hospital after an average of three days without complications.

"This is probably the only kind of donor where the donor actually has some health benefit from donation," Dr. Benedetti said, since losing the portion of bowel typically reduced cholesterol levels.

With these factors in mind, Crook's family approached Dr. Benedetti.

"They came to me ready to have the entire bowel removed," said Dr. Benedetti, "and then, if there was no evidence of recurrence, we would perform a live-donor bowel transplant."

On surgical exploration, Dr. Benedetti and his colleague, Jose Oberholzer, M.D., found a huge tumor about 25 cm by 25 cm occupying nearly the entire abdominal cavity and surrounding the right inferior vena cava.

"But I noticed there was a loop of bowel that was not involved in the tumor," he said. "I immediately thought I could do exactly the same as I am doing for a living donor."

Using the same technique as in live-donor transplant, he removed the three-foot segment of small intestine with its vascular pedicle, flushed it, and laid it aside in a cooling solution for transplant.

He was also able to spare about two feet of proximal bowel by the duodenum to which the segment of small intestine was grafted. By the end of the eight-hour surgery, Crook was left with a total of 120 to 130 cm of bowel.

Surgery for a tumor of that size and location typically leaves a patient without any functioning bowel and in need of intravenous feeding, he added.

After the auto-transplant, Crook recovered quickly and was off parenteral nutrition in about two weeks. Although there was some risk that the transplant would not survive, the risk was lower than it would have been for a living-donor transplant.

"Since the patient is 60," Dr. Benedetti said, "maintaining his own bowel is a tremendous benefit because it avoids the need for long-term immunosuppression."

Desmoid tumors are the ideal indication for such a procedure because they do not present a metastasis risk, he said. "The limitation, of course, is you don't want to shortchange the patient in the cancer ablation."

While transplantation would thus never be considered for malignant tumors, he suggested the procedure could be used for gastrointestinal stromal tumors (GIST).

"I believe that this strategy could be very beneficial for patients with similar disease," he said. "With some decent luck we should be able to find a segment of bowel in most of the situations."

Reviewed by Zalman S. Agus, MD Emeritus Professor University of Pennsylvania School of Medicine

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